Peripheral neuropathy is usually subtle or subclinical in patients with multiple system atrophy (MSA). A 54-year-old man who initially presented with sensory polyneuropathy developed extrapyramidal and cerebellar signs and autonomic failure typical of MSA 7 years later. Sural nerve biopsy disclosed a prominent loss of large myelinated fibers, whereas the unmyelinated fibers were totally spared. These findings imply that sensory neuropathy can be part of the multiple-site degeneration in MSA, and that predominant involvement of large myelinated fibers of the sensory nerves can be a presenting feature.